- Email: [email protected]
Anterior mediastinal nonseminomatous germ cell tumor with malignant transformation: A case report
ORIGINAL REPORTS
Anterior Mediastinal Nonseminomatous Germ Cell Tumor with Malignant Transformation: A Case Report Capt Michael Michel, USAF, MC, and Maj Jerry W. Pratt, USAF, MC Division of Cardiothoracic Surgery, Keesler Medical Center, Keesler Air Force Base, Biloxi, Mississippi OBJECTIVE: We report a case of a 21-year-old man who presented with the unusual symptoms of heart failure and was found to have an anterior mediastinal yolk sac tumor. METHODS: A review of the literature using the Ovid search
engine was performed. RESULTS: The patient was treated with the current standard
of neoadjuvant chemotherapy: bleomycin, etoposide, and cisplatin (BEP) with marked reduction in tumor size, followed by en bloc surgical resection. The final pathology revealed teratoma with malignant change: chondrosarcoma, adenocarcinoma, and poorly differentiated sarcoma. CONCLUSIONS: This is a rare initial presentation of an an-
terior mediastinal germ-cell tumor with treatment consisting of neoadjuvant therapy and surgical resection. In addition, we present the adverse and extremely rare malignant degeneration of this tumor. (Curr Surg 61:576-579. © 2004 by the Association of Program Directors in Surgery.) KEY WORDS: anterior mediastinal germ-cell tumor, malig-
nant degeneration, nonseminomatous, surgical treatment, prognosis
INTRODUCTION Primary neoplasms of the anterior mediastinum are a rare occurrence; nonseminomatous germ cell tumors (NSGCT) account for a small percentage of these. In a review of 702 cases of primary anterior mediastinal tumors in adults, 47% were thymic tumors (primarily thymomas), 22% were lymphomas, 16% were endocrine tumors, and 15% were germ-cell tumors.1
Correspondence: Inquiries to Capt Michael Michel, USAF, MC, Department of Cardiovascular Surgery, Keesler Medical Center, Keesler AFB, MS 39534; fax: (228) 377-6331; e-mail: [email protected] Presented and awarded the Surgeon General’s Award for the Thoracic Surgery Scientific Session at the 50th Annual Symposium of the Society of Air Force Clinical Surgeons. The opinions and assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the United States Government, the Department of Defense, or the United States Air Force.
576
Each of these clinical entities varies in their presentation, diagnosis, treatment, and prognosis. Thymoma may present with symptoms of a paraneoplastic syndrome, such as myasthenia gravis, hypogammaglobulinemia, or pure red cell aplasia; is usually diagnosed with fine needle aspiration (FNA) biopsy; and is generally cured with complete surgical excision.2 Lymphoma should be diagnosed with excisional biopsy but is treated with chemotherapy.3 Germ cell tumors represent a spectrum of the disease process ranging from benign encapsulated masses to extremely aggressive invasive neoplasms. This case report illustrates an unusual presentation of a rare benign primary mediastinal germ cell tumor, which after appropriate neoadjuvant treatment, progressed to malignant degeneration with metastases.
CASE REPORT A previously healthy 20-year-old Hispanic man presented to the Emergency Department with complaints of 1 week of intermittent nausea, fatigue, orthopnea, and minimal dysphagia. The remainder of his medical history was unremarkable. His physical examination revealed a generally healthy appearing young man with no signs of superior vena cava syndrome, no lymphadenopathy, and no abnormalities on genital examination. Significant positive findings included distant heart sounds as well as bilateral lower extremity edema. A chest film demonstrated a markedly increased perihilar density. Computed tomography (CT) scan (Fig. 1) demonstrated a 14 ⫻ 10 ⫻ 18-cm mass located in the anterior mediastinum with compression of the heart and great vessels. In addition, a large pericardial effusion was noted, for which pericardiocentesis was performed. Pertinent laboratory values include an alpha-fetoprotein level of 2098 ng/ml (normal range: 0 to 8 ng/ml), a negative beta-Human Chorionic Gonadotropin (-HCG), and a lactate dehydrogenase (LDH) level of 449 IU/l (normal range: 98 to 192 IU/l). A CT guided needle core biopsy demonstrated cells growing in a reticular pattern with SchillerDuval bodies and eosinophilic droplets (Fig. 2) characteristic of yolk sac tumor.4 Neo-adjuvant treatment was started with the standard regi-
CURRENT SURGERY • © 2004 by the Association of Program Directors in Surgery Published by Elsevier Inc.
0149-7944/04/$30.00 doi:10.1016/j.cursur.2004.05.021
FIGURE 1. Initial CT scan demonstrates complex cystic mass of the anterior mediastinum measuring 14 ⫻ 10 ⫻ 18 cm.
men of 4 cycles of Cisplatin, Etopiside, and Bleomycin (PEB)5,6. After the 4 cycles of PEB, tumor markers had completely normalized and a follow-up CT scan showed a reduction in tumor size to 10 ⫻ 5 ⫻ 11 cm. After chemotherapy, the patient was taken to the operating room where a median sternotomy was performed. The tumor was adherent to the anterior aspect of the pericardium and middle lobe of the right lung, and it encompassed both the right and left phrenic nerves. The majority of the tumor was resected en-bloc, to include a portion of the right middle lobe, the anterior aspect of the pericardium, and a portion of the right phrenic nerve. The left phrenic nerve was skeletonized, leaving a small amount of residual tissue. Postoperatively, the patient recovered well, his hospital course was unremarkable, and he was discharged home in good condition. Pathologic evaluation of the specimen revealed residual viable germ cell tumor and teratoma with malignant degeneration to chondrosarcoma, adenocarcinoma, and poorly differentiated sarcoma (Figs. 3 and 4). Lymph nodes were negative. Six weeks postoperatively, the patient was doing well clini-
FIGURE 2. Initial biopsy specimen demonstrates Schiller-Duval bodies and eosinophilic hyaline droplets.
FIGURE 3. Teratoma with malignant change (chondrosarcoma).
cally and tumor markers remained normal; however, a CT scan revealed new nodules in both the right and left upper lung fields. Despite 2 rounds of salvage chemotherapy with ifosfamide, mesna, cisplatin, and paclitaxel, the tumor continued to aggressively metastasize throughout the chest and abdomen. The patient died 9 months after his initial presentation (5 months after resection).
DISCUSSION The anterior mediastinum is the most common extragonadal site for germ cell tumors.7 These tumors account for only 5% of the 7000 germ cell tumors identified each year in the United States,8with approximately 85% benign. Germ cell neoplasms are classified as either seminomatous or nonseminomatous. Nonseminomatous are further subclassified into embryonal, yolk sac (endodermal sinus), teratocarcinoma, choriocarcinoma, or mixed. Overall, 15% of germ cell tumors have malignant potential, the majority of which contain mixed components; however, rarely do these tumors actually undergo malignant degeneration. In a study by Vuky et al that accumulated 49 patients with primary mediastinal nonseminomatous germ cell tumors over a 20-year period, 32 underwent surgical
FIGURE 4. Teratoma with malignant change (adenocarcinoma and poorly differentiated sarcoma).
CURRENT SURGERY • Volume 61/Number 6 • November/December 2004
577
resection within 3 months after completion of chemotherapy. Of these 32, only 6 were noted to have malignant transformation.9 In addition to harboring an extremely rare tumor, our patient presented with the very unusual symptoms of congestive heart failure. Such a presentation may have been the result of cardiac tamponade resulting from the compression by the large tumor and/or the large pericardial effusion. Most patients that present with a mediastinal germ cell tumor will have symptoms of chest pain or pressure, cough, dyspnea, dysphagia, hoarseness, or superior vena cava syndrome. Constitutional symptoms (weight loss, weakness, fever) are also common in patients with nonseminomatous germ cell tumor.10,11 Most (98%) of these patients are men, presenting between the ages of 20 and 35, with a mean age of 28. Racial minorities are more likely to have extragonadal germ cell tumors than testicular germ cell tumors. Serum tumor markers alpha fetoprotein (AFP), Human chorionic gonadotropin (-HCG), and LDH should be obtained. Alpha fetoprotein and LDH are frequently elevated (80% of patients), whereas elevated AFP is never seen in pure mediastinal seminoma.10 Beta-Human Chorionic Gonadotropin is elevated in 30% to 35% of patients with nonseminomatous tumors. Fine needle aspiration or needle core biopsy will usually confirm the diagnosis in patients with elevated tumor markers. Rarely an anterior mediastinotomy (Chamberlain procedure) may be required for diagnosis. Formal resection should not be undertaken initially as this may delay chemotherapy.12 Several syndromes have been identified in association with nonseminomatous mediastinal germ cell tumors that may aid in diagnosis. Klinefelter’s syndrome, for reasons unknown, is a well-recognized associated syndrome; HCG levels are typically elevated in these cases. Interestingly, the average age at presentation for these patients is 10 years younger than those without Klinefelter’s (18 vs. 28).10 Hematologic neoplasms to include acute myeloid leukemia, acute nonlymphocytic leukemia, acute megakaryocytic leukemia, erythroleukemia, myelodysplastic syndrome, and malignant histiocytosis have also been associated with NSGCTs. This association portends a poor prognosis; Hartmann et al13 reported the median survival after diagnosis of the hematologic malignancy was only 5 months. The current standard of treatment for NSGCT is chemotherapy followed by surgical resection of persistent disease. Radiation therapy has not been shown to be effective in the treatment of primary mediastinal NSGCTs.14,15 Although mediastinal seminomas are sensitive to radiation and small localized tumors may be treated with primary surgical resection and radiotherapy, this approach is not recommended for NSGCTs.11,16 Germ cell tumors, in general, respond extremely well to cisplatin-based combination chemotherapy; 95% of patients, including 80% with metastatic disease at diagnosis, are cured. However, primary mediastinal NSGCTs are not as readily cured. Approximately 50% of patients with disease confined to the mediastinum and only 25% with pulmonary or other visceral metastases will be cured.5 Various combinations of chemotherapy have been tried using cisplatin, vinblastine, 578
bleomycin, etoposide, as well as many other chemotherapeutic agents. Currently, the recommended therapy for primary mediastinal NSGCTs is 4 courses of bleomycin, etoposide, and cisplatin. Bleomycin is generally limited to 10 weeks due to the extensive surgery that will follow chemotherapy and the known pulmonary toxicity of bleomycin.5 Bleomycin toxicity can be monitored with measurement of the diffusion capacity of carbon monoxide, and it should be performed preoperatively to assess the risk of resection. Almost all (⬎95%) patients with mediastinal NSGCT will require resection of residual tumor after chemotherapy. To reduce the risk of postoperative pulmonary failure, intravenous fluids and inspired oxygen concentration should be minimized perioperatively. Mediastinotomy is the most common incision used in these cases; however, depending on size, location, and involvement with other structures, it may be more advantageous to employ a bilateral thoracosternotomy (clamshell incision) or a posterolateral thoracotomy.12 Several factors affect prognosis. Based on a recent Indiana University series, the pathologic findings of the residual mass had the greatest impact on survival. Complete tumor necrosis was associated with excellent survival and no deaths due to recurrent disease. Viable germ cell tumor predicted poor survival with a mean survival of 52 months. Furthermore, multivariate analysis suggested 3 independent predictors of death: (1) persistent germ cell tumor in the residual mass, (2) sarcoma degeneration, and (3) post-chemotherapy AFP level greater that 1001 ng/ml.17 In conclusion, nonseminomatous germ cell tumors of the anterior mediastinum are extremely rare. These tumors can present in an atypical fashion, such as congestive heart failure and have the potential for malignant transformation and metastasis even after appropriate neoadjuvant chemotherapy and surgical resection.
REFERENCES 1. Mullen B, Richardson JD. Primary anterior mediastinal
tumors in children and adults. Ann Thorac Surg. 1986;42: 338-345. 2. Thomas CR, Wright CD, Loehrer PJ. Thymoma: state of
the art. J Clin Oncol. 1999;17:2280-2289. 3. Robinson LA, Dobson JR, Bierman PJ. Fallibility of
transthoracic needle biopsy of anterior mediastinal masses. Thorax. 1995;50:1114-1116. 4. Moran CA, Suster S. Yolk sac tumors of the mediastinum
with prominent spindle cell features: a clinicopathologic study of three cases. Am J Surg Pathol. 1997;21:1173-1177. 5. Einhorn LH. Chemotherapeutic and surgical strategies for
germ cell tumors. Chest Surg Clin N Am. 2002;12:695-706. 6. Strollo DC, Rosado de Christenson ML, Jett JR. Primary
CURRENT SURGERY • Volume 61/Number 6 • November/December 2004
mediastinal tumors. Part 1: tumors of the anterior mediastinum. Chest. 1997;112:511-522.
13. Hartmann JT, Nichols CR, Droz JP, et al. Hematologic
disorders associated with primary mediastinal nonseminomatous germ cell tumors. J Natl Cancer Inst. 2000;92(1): 54-61.
7. Nichols CR. Mediastinal germ cell tumors: clinical fea-
tures and biologic correlates. Chest. 1991;99:472-479. 8. Bosl GJ, Motzer RJ. Testicular germ-cell cancer. N Engl
14. Wright CD, Kesler KA, Nichols CR, et al. Primary medi-
astinal nonseminomatous germ cell tumors. Results of a multimodality approach. J Thoracic Cardiovasc Surg. 1990;99:210-217.
J Med. 1997;337:242-253. 9. Vuky J, Bains M, Bacik J, et al. Role of postchemotherapy
adjunctive surgery in the management of patients with nonseminoma arising from the mediastinum. J Clin Oncol. 2001;19:682-688. 10. Hainsworth JD. Diagnosis,staging, and clinical character-
istics of the patient with mediastinal germ cell carcinoma. Chest Surg Clin N Am. 2002;12:665-672. 11. Weidner N. Germ cell tumors of the mediastinum. Semin
Diagn Pathol. 1999;16(1):42-50.
15. Kersh CR, Eisert DR, Constable WC, et al. Primary ma-
lignant mediastinal germ-cell tumors and the contribution of radiotherapy: a southeastern multi-institutional study. Am J Clin Oncol. 1987;10:302-306. 16. Bukowski RM, Wolf M, Kulander BG, et al. Alternating
combination chemotherapy in patients with extragonadal germ cell tumors. Cancer. 1993;71:2631-2638. 17. Kesler KA, Reiger KM, Ganjoo KN, et al. Primary
12. Wright CD, Kesler KA. Surgical techniques and outcomes
for primary nonseminomatous germ cell tumors. Chest Surg Clin N Am. 2002;12:707-715.
CURRENT SURGERY • Volume 61/Number 6 • November/December 2004
mediastinal nonseminomatous germ cell tumors: the influence of postchemotherapy pathology on long-term survival after surgery. J Thorac Cardiovasc Surg. 1999; 118:692-701.
579
Anterior Mediastinal Nonseminomatous Germ Cell Tumor with Malignant Transformation: A Case Report Capt Michael Michel, USAF, MC, and Maj Jerry W. Pratt, USAF, MC Division of Cardiothoracic Surgery, Keesler Medical Center, Keesler Air Force Base, Biloxi, Mississippi OBJECTIVE: We report a case of a 21-year-old man who presented with the unusual symptoms of heart failure and was found to have an anterior mediastinal yolk sac tumor. METHODS: A review of the literature using the Ovid search
engine was performed. RESULTS: The patient was treated with the current standard
of neoadjuvant chemotherapy: bleomycin, etoposide, and cisplatin (BEP) with marked reduction in tumor size, followed by en bloc surgical resection. The final pathology revealed teratoma with malignant change: chondrosarcoma, adenocarcinoma, and poorly differentiated sarcoma. CONCLUSIONS: This is a rare initial presentation of an an-
terior mediastinal germ-cell tumor with treatment consisting of neoadjuvant therapy and surgical resection. In addition, we present the adverse and extremely rare malignant degeneration of this tumor. (Curr Surg 61:576-579. © 2004 by the Association of Program Directors in Surgery.) KEY WORDS: anterior mediastinal germ-cell tumor, malig-
nant degeneration, nonseminomatous, surgical treatment, prognosis
INTRODUCTION Primary neoplasms of the anterior mediastinum are a rare occurrence; nonseminomatous germ cell tumors (NSGCT) account for a small percentage of these. In a review of 702 cases of primary anterior mediastinal tumors in adults, 47% were thymic tumors (primarily thymomas), 22% were lymphomas, 16% were endocrine tumors, and 15% were germ-cell tumors.1
Correspondence: Inquiries to Capt Michael Michel, USAF, MC, Department of Cardiovascular Surgery, Keesler Medical Center, Keesler AFB, MS 39534; fax: (228) 377-6331; e-mail: [email protected] Presented and awarded the Surgeon General’s Award for the Thoracic Surgery Scientific Session at the 50th Annual Symposium of the Society of Air Force Clinical Surgeons. The opinions and assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the United States Government, the Department of Defense, or the United States Air Force.
576
Each of these clinical entities varies in their presentation, diagnosis, treatment, and prognosis. Thymoma may present with symptoms of a paraneoplastic syndrome, such as myasthenia gravis, hypogammaglobulinemia, or pure red cell aplasia; is usually diagnosed with fine needle aspiration (FNA) biopsy; and is generally cured with complete surgical excision.2 Lymphoma should be diagnosed with excisional biopsy but is treated with chemotherapy.3 Germ cell tumors represent a spectrum of the disease process ranging from benign encapsulated masses to extremely aggressive invasive neoplasms. This case report illustrates an unusual presentation of a rare benign primary mediastinal germ cell tumor, which after appropriate neoadjuvant treatment, progressed to malignant degeneration with metastases.
CASE REPORT A previously healthy 20-year-old Hispanic man presented to the Emergency Department with complaints of 1 week of intermittent nausea, fatigue, orthopnea, and minimal dysphagia. The remainder of his medical history was unremarkable. His physical examination revealed a generally healthy appearing young man with no signs of superior vena cava syndrome, no lymphadenopathy, and no abnormalities on genital examination. Significant positive findings included distant heart sounds as well as bilateral lower extremity edema. A chest film demonstrated a markedly increased perihilar density. Computed tomography (CT) scan (Fig. 1) demonstrated a 14 ⫻ 10 ⫻ 18-cm mass located in the anterior mediastinum with compression of the heart and great vessels. In addition, a large pericardial effusion was noted, for which pericardiocentesis was performed. Pertinent laboratory values include an alpha-fetoprotein level of 2098 ng/ml (normal range: 0 to 8 ng/ml), a negative beta-Human Chorionic Gonadotropin (-HCG), and a lactate dehydrogenase (LDH) level of 449 IU/l (normal range: 98 to 192 IU/l). A CT guided needle core biopsy demonstrated cells growing in a reticular pattern with SchillerDuval bodies and eosinophilic droplets (Fig. 2) characteristic of yolk sac tumor.4 Neo-adjuvant treatment was started with the standard regi-
CURRENT SURGERY • © 2004 by the Association of Program Directors in Surgery Published by Elsevier Inc.
0149-7944/04/$30.00 doi:10.1016/j.cursur.2004.05.021
FIGURE 1. Initial CT scan demonstrates complex cystic mass of the anterior mediastinum measuring 14 ⫻ 10 ⫻ 18 cm.
men of 4 cycles of Cisplatin, Etopiside, and Bleomycin (PEB)5,6. After the 4 cycles of PEB, tumor markers had completely normalized and a follow-up CT scan showed a reduction in tumor size to 10 ⫻ 5 ⫻ 11 cm. After chemotherapy, the patient was taken to the operating room where a median sternotomy was performed. The tumor was adherent to the anterior aspect of the pericardium and middle lobe of the right lung, and it encompassed both the right and left phrenic nerves. The majority of the tumor was resected en-bloc, to include a portion of the right middle lobe, the anterior aspect of the pericardium, and a portion of the right phrenic nerve. The left phrenic nerve was skeletonized, leaving a small amount of residual tissue. Postoperatively, the patient recovered well, his hospital course was unremarkable, and he was discharged home in good condition. Pathologic evaluation of the specimen revealed residual viable germ cell tumor and teratoma with malignant degeneration to chondrosarcoma, adenocarcinoma, and poorly differentiated sarcoma (Figs. 3 and 4). Lymph nodes were negative. Six weeks postoperatively, the patient was doing well clini-
FIGURE 2. Initial biopsy specimen demonstrates Schiller-Duval bodies and eosinophilic hyaline droplets.
FIGURE 3. Teratoma with malignant change (chondrosarcoma).
cally and tumor markers remained normal; however, a CT scan revealed new nodules in both the right and left upper lung fields. Despite 2 rounds of salvage chemotherapy with ifosfamide, mesna, cisplatin, and paclitaxel, the tumor continued to aggressively metastasize throughout the chest and abdomen. The patient died 9 months after his initial presentation (5 months after resection).
DISCUSSION The anterior mediastinum is the most common extragonadal site for germ cell tumors.7 These tumors account for only 5% of the 7000 germ cell tumors identified each year in the United States,8with approximately 85% benign. Germ cell neoplasms are classified as either seminomatous or nonseminomatous. Nonseminomatous are further subclassified into embryonal, yolk sac (endodermal sinus), teratocarcinoma, choriocarcinoma, or mixed. Overall, 15% of germ cell tumors have malignant potential, the majority of which contain mixed components; however, rarely do these tumors actually undergo malignant degeneration. In a study by Vuky et al that accumulated 49 patients with primary mediastinal nonseminomatous germ cell tumors over a 20-year period, 32 underwent surgical
FIGURE 4. Teratoma with malignant change (adenocarcinoma and poorly differentiated sarcoma).
CURRENT SURGERY • Volume 61/Number 6 • November/December 2004
577
resection within 3 months after completion of chemotherapy. Of these 32, only 6 were noted to have malignant transformation.9 In addition to harboring an extremely rare tumor, our patient presented with the very unusual symptoms of congestive heart failure. Such a presentation may have been the result of cardiac tamponade resulting from the compression by the large tumor and/or the large pericardial effusion. Most patients that present with a mediastinal germ cell tumor will have symptoms of chest pain or pressure, cough, dyspnea, dysphagia, hoarseness, or superior vena cava syndrome. Constitutional symptoms (weight loss, weakness, fever) are also common in patients with nonseminomatous germ cell tumor.10,11 Most (98%) of these patients are men, presenting between the ages of 20 and 35, with a mean age of 28. Racial minorities are more likely to have extragonadal germ cell tumors than testicular germ cell tumors. Serum tumor markers alpha fetoprotein (AFP), Human chorionic gonadotropin (-HCG), and LDH should be obtained. Alpha fetoprotein and LDH are frequently elevated (80% of patients), whereas elevated AFP is never seen in pure mediastinal seminoma.10 Beta-Human Chorionic Gonadotropin is elevated in 30% to 35% of patients with nonseminomatous tumors. Fine needle aspiration or needle core biopsy will usually confirm the diagnosis in patients with elevated tumor markers. Rarely an anterior mediastinotomy (Chamberlain procedure) may be required for diagnosis. Formal resection should not be undertaken initially as this may delay chemotherapy.12 Several syndromes have been identified in association with nonseminomatous mediastinal germ cell tumors that may aid in diagnosis. Klinefelter’s syndrome, for reasons unknown, is a well-recognized associated syndrome; HCG levels are typically elevated in these cases. Interestingly, the average age at presentation for these patients is 10 years younger than those without Klinefelter’s (18 vs. 28).10 Hematologic neoplasms to include acute myeloid leukemia, acute nonlymphocytic leukemia, acute megakaryocytic leukemia, erythroleukemia, myelodysplastic syndrome, and malignant histiocytosis have also been associated with NSGCTs. This association portends a poor prognosis; Hartmann et al13 reported the median survival after diagnosis of the hematologic malignancy was only 5 months. The current standard of treatment for NSGCT is chemotherapy followed by surgical resection of persistent disease. Radiation therapy has not been shown to be effective in the treatment of primary mediastinal NSGCTs.14,15 Although mediastinal seminomas are sensitive to radiation and small localized tumors may be treated with primary surgical resection and radiotherapy, this approach is not recommended for NSGCTs.11,16 Germ cell tumors, in general, respond extremely well to cisplatin-based combination chemotherapy; 95% of patients, including 80% with metastatic disease at diagnosis, are cured. However, primary mediastinal NSGCTs are not as readily cured. Approximately 50% of patients with disease confined to the mediastinum and only 25% with pulmonary or other visceral metastases will be cured.5 Various combinations of chemotherapy have been tried using cisplatin, vinblastine, 578
bleomycin, etoposide, as well as many other chemotherapeutic agents. Currently, the recommended therapy for primary mediastinal NSGCTs is 4 courses of bleomycin, etoposide, and cisplatin. Bleomycin is generally limited to 10 weeks due to the extensive surgery that will follow chemotherapy and the known pulmonary toxicity of bleomycin.5 Bleomycin toxicity can be monitored with measurement of the diffusion capacity of carbon monoxide, and it should be performed preoperatively to assess the risk of resection. Almost all (⬎95%) patients with mediastinal NSGCT will require resection of residual tumor after chemotherapy. To reduce the risk of postoperative pulmonary failure, intravenous fluids and inspired oxygen concentration should be minimized perioperatively. Mediastinotomy is the most common incision used in these cases; however, depending on size, location, and involvement with other structures, it may be more advantageous to employ a bilateral thoracosternotomy (clamshell incision) or a posterolateral thoracotomy.12 Several factors affect prognosis. Based on a recent Indiana University series, the pathologic findings of the residual mass had the greatest impact on survival. Complete tumor necrosis was associated with excellent survival and no deaths due to recurrent disease. Viable germ cell tumor predicted poor survival with a mean survival of 52 months. Furthermore, multivariate analysis suggested 3 independent predictors of death: (1) persistent germ cell tumor in the residual mass, (2) sarcoma degeneration, and (3) post-chemotherapy AFP level greater that 1001 ng/ml.17 In conclusion, nonseminomatous germ cell tumors of the anterior mediastinum are extremely rare. These tumors can present in an atypical fashion, such as congestive heart failure and have the potential for malignant transformation and metastasis even after appropriate neoadjuvant chemotherapy and surgical resection.
REFERENCES 1. Mullen B, Richardson JD. Primary anterior mediastinal
tumors in children and adults. Ann Thorac Surg. 1986;42: 338-345. 2. Thomas CR, Wright CD, Loehrer PJ. Thymoma: state of
the art. J Clin Oncol. 1999;17:2280-2289. 3. Robinson LA, Dobson JR, Bierman PJ. Fallibility of
transthoracic needle biopsy of anterior mediastinal masses. Thorax. 1995;50:1114-1116. 4. Moran CA, Suster S. Yolk sac tumors of the mediastinum
with prominent spindle cell features: a clinicopathologic study of three cases. Am J Surg Pathol. 1997;21:1173-1177. 5. Einhorn LH. Chemotherapeutic and surgical strategies for
germ cell tumors. Chest Surg Clin N Am. 2002;12:695-706. 6. Strollo DC, Rosado de Christenson ML, Jett JR. Primary
CURRENT SURGERY • Volume 61/Number 6 • November/December 2004
mediastinal tumors. Part 1: tumors of the anterior mediastinum. Chest. 1997;112:511-522.
13. Hartmann JT, Nichols CR, Droz JP, et al. Hematologic
disorders associated with primary mediastinal nonseminomatous germ cell tumors. J Natl Cancer Inst. 2000;92(1): 54-61.
7. Nichols CR. Mediastinal germ cell tumors: clinical fea-
tures and biologic correlates. Chest. 1991;99:472-479. 8. Bosl GJ, Motzer RJ. Testicular germ-cell cancer. N Engl
14. Wright CD, Kesler KA, Nichols CR, et al. Primary medi-
astinal nonseminomatous germ cell tumors. Results of a multimodality approach. J Thoracic Cardiovasc Surg. 1990;99:210-217.
J Med. 1997;337:242-253. 9. Vuky J, Bains M, Bacik J, et al. Role of postchemotherapy
adjunctive surgery in the management of patients with nonseminoma arising from the mediastinum. J Clin Oncol. 2001;19:682-688. 10. Hainsworth JD. Diagnosis,staging, and clinical character-
istics of the patient with mediastinal germ cell carcinoma. Chest Surg Clin N Am. 2002;12:665-672. 11. Weidner N. Germ cell tumors of the mediastinum. Semin
Diagn Pathol. 1999;16(1):42-50.
15. Kersh CR, Eisert DR, Constable WC, et al. Primary ma-
lignant mediastinal germ-cell tumors and the contribution of radiotherapy: a southeastern multi-institutional study. Am J Clin Oncol. 1987;10:302-306. 16. Bukowski RM, Wolf M, Kulander BG, et al. Alternating
combination chemotherapy in patients with extragonadal germ cell tumors. Cancer. 1993;71:2631-2638. 17. Kesler KA, Reiger KM, Ganjoo KN, et al. Primary
12. Wright CD, Kesler KA. Surgical techniques and outcomes
for primary nonseminomatous germ cell tumors. Chest Surg Clin N Am. 2002;12:707-715.
CURRENT SURGERY • Volume 61/Number 6 • November/December 2004
mediastinal nonseminomatous germ cell tumors: the influence of postchemotherapy pathology on long-term survival after surgery. J Thorac Cardiovasc Surg. 1999; 118:692-701.
579