Primary Mediastinal Yolk Sac Tumor Research Articles

A Rare Case of Metastatic Extragonadal Yolk Sac Tumor – A Challenging Diagnosis on Cytomorphology

Abstract Introduction/Objective Yolk sac tumors are characterized by variable histologic patterns and broad cytologic spectrum; thereby making the distinction from a poorly differentiated adenocarcinoma in a metastatic site extremely challenging. We present cytology and histology of a metastatic extragonadal germ cell tumor with predominant solid yolk sac component. Methods/Case Report 36-year-old male presented with bilateral supraclavicular masses (right: 6 x 7 cm, left: 3 x 4 cm). CT chest and abdomen revealed large mediastinal and hilar nodal mass (11.6 cm), right hilar mass (8.5 cm), bilateral heterogeneous adrenal masses (right: 11.4 cm, left: 3.1 cm), and an enhancing retroperitoneal implant. Differential diagnoses of lymphoma, adrenocortical carcinoma, small cell lung carcinoma, and metastatic germ cell tumor were considered. Testicular ultrasound revealed no masses. Fine needle aspiration of supraclavicular lymph nodes showed highly cellular smears comprised of tumor cells, dispersed singly and in clusters, with irregular nuclear contours, increased nuclear to cytoplasmic ratio, coarse chromatin, occasional prominent nucleoli, and vacuolated cytoplasm. Tumor cells were positive for KRT7 and TTF-1, and negative for p40 and PAX8. A diagnosis of metastatic poorly differentiated carcinoma was rendered. Subsequent excisional biopsy of the lymph node showed a metastatic tumor with tumor cells similar to those seen in cytology, appearing in solid sheets with focal papillary architecture, replacing the entire lymph node. Additional immunohistochemistry showed expression of SALL4, Glypican 3, with lack of expression for OCT3/4, AFP, hCG, and CD30, supporting the diagnosis of metastatic yolk sac tumor, solid pattern. Results (if a Case Study enter NA) NA. Conclusion Aggressive clinical presentation, broad clinical differential diagnoses, rarity of a primary mediastinal yolk sac tumor, non-diagnostic cytomorphology along with expression of divergent differentiation markers (TTF-1 in our case) makes a definitive diagnosis of metastatic extragonadal yolk sac tumor extremely challenging. High index of clinical suspicion is required for a definitive diagnosis on cytomorphology.

The Role of Surgical Resection as A Salvage Therapy and Immunohistochemical Stain to Confirm Mediastinal Yolk Sac Tumor

Background: Mediastinal germ cell tumors are uncommon, representing less than 1% of all malignancies. They mostly occur in young men after puberty and have a poor prognosis with over all long term survival rate 42%. Case Report: We reported a case report in Saiful Anwar Malang Hospital, Indonesia, a young man, 26 years old with an anterior mediastinum tumor and VCSS The CT scan of the chest showed an anterosuperior mediastinal mass which compress the superior vena cava. The result from transthoracic FNAB with USG guidance showed an extragonadal germ cell tumor which resemble an Embryonal Carcinoma from mediastinum. The AFP serum level was elevated. Patient underwent a radiotherapy and a surgical debulking. The biopsy showed an Endodermal Sinus Tumor (Yolk Sac). Immunohistochemical stain for cytokeratin was positive in the tumor cells while stain for CD30 was negative. These were confirming the diagnosis of primary mediastinal yolk sac tumor. Patient received a combination chemotherapy for a total 6 cycles.Conclusion: Some procedures like biopsy, tumor marker, immunohistochemical stain, could differentiate the subtype of mediastinal germ cell tumor. The multimodality treatment by combining chemotherapy with surgical therapy or radiotherapy could increase the survival outcome of patients.

Diagnosis and treatment of seven primary mediastinal yolk sac tumors

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. 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. 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 experienced 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. 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.

A case of primary mediastinal yolk sac tumor completely resected by video-assisted thoracic surgery and median sternotomy

A case of primary mediastinal yolk sac tumor completely resected by video-assisted thoracic surgery and median sternotomy

Tumor Mediastinum Anterior ( Yolk Sac Tumor ) pada Seorang Laki-Laki Dewasa Muda: Sebuah Kasus yang Jarang

Background: Primary mediastinal yolk sac tumor is an extremely rare and highly malignant tumors occuring in children and young adult. They are more common in men. The most common symptoms on ptesentation were dyspnea, chest pain, cought, fever, night sweat, or weight loss. Primary mediastinal tumor are considered to have poor prognosis. Case: A 18- year-old man who presented with shorthness of breath, chest pain, fever, night sweat, and generalized weakness. He had no significant surgical, familiy or social history. The chest X-Ray and computed tomographic scan of the chest showed a large anterior mediasti nal mass. The serum level of alpha- fetoprotein and β-HCG was elevated. The histological examination revealed the finding of yolk sac tumor. This supported the diagnosis of yolk sac tumor. The patient received a combination chemotherapy consisting of cisplatin, etoposide and bleomycin every 3 weeks for total of 4 cycles. Conclusions: Primary mediastinal yolk sac tumor is a rare tumor. The diagnosis should be made not only by morphological studies but the also the patients age and the elevation of serum alpha-fetoprotein. In spite of modern chemotherapy, the prognosis of mediastinal yolk sac tumor remains poor.

Chemotherapy for primary mediastinal yolk sac tumor in a patient undergoing chronic hemodialysis: a case report

BackgroundThe safety and efficacy of chemotherapy for patients undergoing concomitant hemodialysis have not been fully established and optimal doses of anti-cancer drugs and best timing of hemodialysis remains unclear. Although chemosensitive cancers, such as germ cell tumors, treated with chemotherapy should have sufficient dose intensity maintained to achieve the desired effect, many patients with cancer undergoing hemodialysis might be under-treated because the pharmacokinetics of anti-cancer drugs in such patients remains unknown.Case presentationWe describe a 31-year-old Japanese man with a mediastinal yolk sac tumor treated with surgery followed by five cycles of chemotherapy containing cisplatin and etoposide while concomitantly undergoing hemodialysis. The doses of these agents used in the first cycle were 50% of the standard dose of cisplatin (10 mg/m2) and 60% of the standard dose of etoposide (60 mg/m2) on days 1 through to 5; the doses were subsequently escalated to 75% with both agents. Hemodialysis was started 1 hour after infusions of these agents. Severe hematological toxicities were observed despite successful treatment. During treatment with concurrent hemodialysis, pharmacokinetic analysis of cisplatin was performed and its relationship with adverse effects was assessed. Compared with patients with normal renal function, the maximum drug concentration was higher, and concentration increased in the interval between hemodialysis and the subsequent cisplatin infusion, resulting in a higher area under the curve despite a reduction in the dose to 75% of the standard regimen.ConclusionsBecause of the altered pharmacokinetics pharmacodynamics status of patients with renal dysfunction undergoing hemodialysis, pharmacokinetics pharmacodynamics analysis is deemed to be helpful for effective and safe management of chemotherapy in patients undergoing hemodialysis.

Angiosarcoma After Treatment of Primary Mediastinal Yolk Sac Tumor

SESSION TITLE: Student/Resident Case Report Poster - Disorders of the Mediastinum SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Germ cell tumors are often composed of several different cell types. Rarely, these tumors can include sarcomatous components, such as rhabdomyosarcoma or angiosarcoma, which may be realized after failure of chemotherapy to reduce tumor size. Currently, given the poor response rate of systemic therapy for angiosarcoma, surgical resection is considered the most effective therapy. Monitoring response to therapy with tumor markers and imaging is important, as it may identify chemo-refractory malignancies. CASE PRESENTATION: A 54-year-old male without prior medical history presented with dyspnea and 10-pound weight loss. His initial imaging identified a 5x13x8 cm anterior mediastinal mass; labs were notable for an elevated alpha fetoprotein (AFP). Biopsy of the mass revealed a yolk sac tumor. He was received four cycles of combination chemotherapy for non-seminomatous germ cell tumor with etoposide, ifosfamide, and cisplatin. Despite normalization of the AFP level, a repeat CT scan revealed that the mediastinal mass had increased in size. A repeat biopsy of the anterior mediastinum showed angiosarcoma; biopsy of a sacral lesion detected on SPECT scan also showed angiosarcoma, consistent with metastatic disease. The mediastinal tumor was partially resected, and the patient was subsequently initiated on paclitaxel. DISCUSSION: This case demonstrates the importance of monitoring for progression of germ cell tumors with serial imaging, in addition to tumor markers. Similar to other case reports, this patient experienced normalization of his tumor markers, but demonstrated increasing tumor size on imaging. Detecting the expanding tumor prompted a concern for the presence tumor cells that were unaffected by treatment, which, in this case, were revealed to be those of angiosarcoma. Because angiosarcomas are very unlikely to respond to chemotherapy, the prognosis is poor. Novel therapies are under investigation. CONCLUSIONS: Imaging is necessary in addition to tumor markers to identify unexpected outcomes in the course of treatment of germ cell tumors. Reference #1: Mediastinal Germ Cell Tumors with an Angiosarcomatous Component: A Report of 12 Cases. Alejandro Luina Contreras, MD et al. Hum Pathol. 2010 June; 41 (6): 832-837 Reference #2: Poor prognosis of mediastinal germ cell cancers containing sarcomatous components. Gonzalez-Vela et al. Cancer. 1990 Sep 15;66(6): 1114-6. Reference #3: Germ cell tumors with sarcomatous components: a clinicopathologic and immunohistochemical study of 46 cases. Malagon HD, Valdez AM, Moran CA, Suster S. Am J Surg Pathol. 2007 Sep;31(9):1356-62 DISCLOSURE: The following authors have nothing to disclose: Huy Do, Janine Vintch, Yvonne Carter, James Yeh, Justin Darrah No Product/Research Disclosure Information

A Rare Case of Primary Anterior Mediastinal Yolk Sac Tumor in an Elderly Adult Male.

Mediastinal germ cell tumors are extragonadal germ cell tumors (EGGCTs) commonly seen in children and young adults. They are more common in men. Clinically they are classified as teratomas, seminomas, and nonseminomatous germ cell tumors. Primary mediastinal yolk sac neoplasm is an extremely rare tumor. We present here a very rare case of primary yolk sac tumor of the anterior mediastinum in a 73-year-old male. Mediastinal germ cell tumors have a worse prognosis than gonadal germ cell tumors. Chemotherapy followed by adjuvant surgery improves overall response in EGGCTs. However, comorbidities can render treatment with chemotherapy and surgery challenging in elderly patients.

Successful radiotherapy in postoperative recurrence of a primary mediastinal yolk sac tumor: A case report.

A woman in her 60s was evaluated for anterior chest pain. Computed tomography (CT) revealed a 50 mm mass with irregular contrast enhancement in the anterior mediastinum. α‐fetoprotein (AFP) level was elevated to 1188 ng/mL. A germ cell tumor was diagnosed, mostly comprising of a yolk sac tumor (YST). Two courses of chemotherapy with cisplatin (CDDP) and etoposide (VP16) were administered and surgical tumor resection was then performed. The final diagnosis was YST. CDDP and VP16 were continued postoperatively; however, because the AFP level increased about six months after surgery, the chemotherapy regimen was altered to bleomycin and CPT‐11. As the AFP again increased and a CT scan revealed tumor re‐enlargement, recurrent YST was diagnosed and radiotherapy was administered. The patient received a total of 60 Gy (2 Gy per fraction). The tumor started to shrink during radiotherapy and AFP levels decreased. By one month post‐radiotherapy, AFP levels had normalized and the tumor had disappeared. As of six years after radiotherapy, the patient remains alive without recurrence. Mediastinal YSTs are rare, and treatment usually includes surgery and preoperative and postoperative chemotherapy with cisplatin‐based regimens. Successful treatment with radiotherapy has occasionally been reported. Our patient showed recurrence of a YST after surgery and chemotherapy, but achieved long‐term survival after radiotherapy. Few patients with YST have undergone radiotherapy, but this approach was successful in our patient. In cases of postoperative recurrent YST resistant to chemotherapy, radiotherapy, together with salvage surgery, may offer a valuable option.

A rare case of primary mediastinal yolk sac tumor

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.