Malignant mediastinal germ cell tumors

An intergroup study

Deborah Billmire, Charles Vinocur, Frederick Rescorla, Paul Colombani, Barbara Cushing, Edith Hawkins, Wendy B. London, Roger Giller, Steve Lauer

Research output: Contribution to journalArticle

75 Citations (Scopus)

Abstract

Purpose: This review was conducted to determine clinical characteristics and response to therapy in this rare pediatric neoplasm. Methods: An intergroup Pediatric Oncology Group (POG) 9049/Children's Cancer Study Group (CCG) 8882 randomized trial was conducted to evaluate response rate and survival with chemotherapy using etoposide, bleomycin, and high or standard dose cisplatin for high-risk malignant germ cell tumors at extragonadal sites. For this review, a secondary analysis of clinical and operative findings in patients with primary site in the mediastinum was carried out. Results: Of the 38 children with malignant mediastinal germ cell tumors (MGCT), 36 had sufficient data to be included in this review. Thirty-four tumors were anterior mediastinal, 2 were intrapericardial. Younger patients had respiratory complaints; older patients had chest pain, precocious puberty, or facial fullness. Yolk sac tumor was the only malignant element in girls. Boys had yolk sac tumor in 7, germinoma in 3, choriocarcinoma in 2, and mixed malignant elements in 15. Benign teratoma elements coexisted in 22 patients. Four patients had biopsy and chemotherapy without tumor resection, and only 1 survived. Fourteen patients had resection at diagnosis followed by chemotherapy with 12 survivors. Eighteen patients had biopsy followed by chemotherapy and postchemotherapy tumor resection with 13 survivors. Tumor size in response to chemotherapy for these 18 patients was stable or increased in 6, and decreased in 12 (mean decrease of 57% in greatest dimension). Overall, 26 of 36 patients survived, with a 4-year patient survival rate of 71% ± 10%, and a 4-year event-free survival rate of 69% ± 10%. Ten patients died: 5 of tumor (all boys ≥15 yr), 2 of sepsis, and 3 of second malignancy. Conclusions: Malignant MGCT is a complex tumor of varied histology with frequent coexistence of benign elements. Lesions often have incomplete regression with chemotherapy alone. Tumor resection may be undertaken at diagnosis or after attempted shrinkage with chemotherapy. Aggressive attempt at complete tumor resection should be offered to all patients even if bulky tumor persists after induction chemotherapy with expectation of a significant salvage rate. Boys ≥15 years may be a high-risk subgroup for mortality from tumor progression.

Original languageEnglish (US)
Pages (from-to)18-24
Number of pages7
JournalJournal of Pediatric Surgery
Volume36
Issue number1
DOIs
StatePublished - 2001
Externally publishedYes

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Germ Cell and Embryonal Neoplasms
Neoplasms
Drug Therapy
Endodermal Sinus Tumor
Survival Rate
Survivors
Germinoma
Pediatrics
Biopsy
Precocious Puberty
Choriocarcinoma
Induction Chemotherapy
Second Primary Neoplasms
Teratoma
Bleomycin
Mediastinum
Etoposide
Chest Pain
Cisplatin
Disease-Free Survival

Keywords

  • Choriocarcinoma
  • Germinoma
  • Malignant mediastinal germ cell tumors
  • Yolk sac tumor

ASJC Scopus subject areas

  • Surgery

Cite this

Billmire, D., Vinocur, C., Rescorla, F., Colombani, P., Cushing, B., Hawkins, E., ... Lauer, S. (2001). Malignant mediastinal germ cell tumors: An intergroup study. Journal of Pediatric Surgery, 36(1), 18-24. https://doi.org/10.1053/jpsu.2001.19995

Malignant mediastinal germ cell tumors : An intergroup study. / Billmire, Deborah; Vinocur, Charles; Rescorla, Frederick; Colombani, Paul; Cushing, Barbara; Hawkins, Edith; London, Wendy B.; Giller, Roger; Lauer, Steve.

In: Journal of Pediatric Surgery, Vol. 36, No. 1, 2001, p. 18-24.

Research output: Contribution to journalArticle

Billmire, D, Vinocur, C, Rescorla, F, Colombani, P, Cushing, B, Hawkins, E, London, WB, Giller, R & Lauer, S 2001, 'Malignant mediastinal germ cell tumors: An intergroup study', Journal of Pediatric Surgery, vol. 36, no. 1, pp. 18-24. https://doi.org/10.1053/jpsu.2001.19995
Billmire, Deborah ; Vinocur, Charles ; Rescorla, Frederick ; Colombani, Paul ; Cushing, Barbara ; Hawkins, Edith ; London, Wendy B. ; Giller, Roger ; Lauer, Steve. / Malignant mediastinal germ cell tumors : An intergroup study. In: Journal of Pediatric Surgery. 2001 ; Vol. 36, No. 1. pp. 18-24.
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AU - Billmire, Deborah

AU - Vinocur, Charles

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AU - Hawkins, Edith

AU - London, Wendy B.

AU - Giller, Roger

AU - Lauer, Steve

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N2 - Purpose: This review was conducted to determine clinical characteristics and response to therapy in this rare pediatric neoplasm. Methods: An intergroup Pediatric Oncology Group (POG) 9049/Children's Cancer Study Group (CCG) 8882 randomized trial was conducted to evaluate response rate and survival with chemotherapy using etoposide, bleomycin, and high or standard dose cisplatin for high-risk malignant germ cell tumors at extragonadal sites. For this review, a secondary analysis of clinical and operative findings in patients with primary site in the mediastinum was carried out. Results: Of the 38 children with malignant mediastinal germ cell tumors (MGCT), 36 had sufficient data to be included in this review. Thirty-four tumors were anterior mediastinal, 2 were intrapericardial. Younger patients had respiratory complaints; older patients had chest pain, precocious puberty, or facial fullness. Yolk sac tumor was the only malignant element in girls. Boys had yolk sac tumor in 7, germinoma in 3, choriocarcinoma in 2, and mixed malignant elements in 15. Benign teratoma elements coexisted in 22 patients. Four patients had biopsy and chemotherapy without tumor resection, and only 1 survived. Fourteen patients had resection at diagnosis followed by chemotherapy with 12 survivors. Eighteen patients had biopsy followed by chemotherapy and postchemotherapy tumor resection with 13 survivors. Tumor size in response to chemotherapy for these 18 patients was stable or increased in 6, and decreased in 12 (mean decrease of 57% in greatest dimension). Overall, 26 of 36 patients survived, with a 4-year patient survival rate of 71% ± 10%, and a 4-year event-free survival rate of 69% ± 10%. Ten patients died: 5 of tumor (all boys ≥15 yr), 2 of sepsis, and 3 of second malignancy. Conclusions: Malignant MGCT is a complex tumor of varied histology with frequent coexistence of benign elements. Lesions often have incomplete regression with chemotherapy alone. Tumor resection may be undertaken at diagnosis or after attempted shrinkage with chemotherapy. Aggressive attempt at complete tumor resection should be offered to all patients even if bulky tumor persists after induction chemotherapy with expectation of a significant salvage rate. Boys ≥15 years may be a high-risk subgroup for mortality from tumor progression.

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