Primary Mediastinal Germ Cell Tumors Research Articles

A case of mediastinal germ cell tumor radically operated on after neoadjuvant chemotherapy-combined resection of the superior vena cava and reconstruction with expanded-PTFE graft

A case of primary mediastinal germ cell tumor, which was radically treated with reconstruction of the superior vena cava (SVC) after neoadjuvant chemotherapy, is reported herein. This tumor, accompanied with SVC syndrome, was initially unresectably advanced and chemotherapy, using cis-dichlorodiamine-platinum, vindesine and bleomycin, was administered, resulting in a partial response with a 65 per cent tumor regression. The serum human chorionic gonadotropin, which originally had a high value, became reduced in parallel with the tumor regression to within the normal range. Complete tumor resection with reconstruction of the SVC using an expanded polytetrafluoroethylene (EPTFE) graft was successfully performed. The patient has now returned to productive employment and the graft is patent without any clinical symptoms of recurrence, 15 months post-operatively. Neoadjuvant chemotherapy is considered to be vitally important for the treatment of germ cell tumors originating in the anterior mediastinum and an EPTFE graft with external ring support seems to be acceptable as an artificial substitute for SVC replacement.

Klinefelter's syndrome associated with mediastinal germ cell neoplasms.

Several case reports have suggested an association of primary mediastinal germ cell tumor (PMGCT) and Klinefelter's syndrome (KS). In an effort to confirm this association, 22 patients with mediastinal germ cell tumors had chromosome studies performed in a prospective fashion. Five patients (22%) had karyotypic or pathologic evidence of KS. All of the patients with KS had germ cell tumors of the nonseminomatous subtype and were relatively young (median age, 15 years). The literature confirms the findings of a young median age (18 years), nonseminomatous subtype, and mediastinal location of the germ cell neoplasm. We conclude that patients with KS are predisposed to the development of mediastinal nonseminomatous germ cell cancers.

Mediastinal germ cell tumors in childhood: A clinical and pathological study of 21 cases

Twenty-one patients aged 16 years or less had been treated for a primary mediastinal germ cell tumor at the Children's Hospital, Boston Massachusetts, during the last 54 years. There were 13 boys and eight girls with the average age at diagnosis being 7 years (range 2 weeks to 16 years). Twelve mediastinal germ cell tumors were classified as pure teratoma, five contained embryonal carcinoma admixed with other germ cell components, and four were pure embryonal carcinoma. Of 12 patients with pure teratoma, 10 underwent complete surgical resection and were alive and well 1 to 13 years later; two children left untreated died of complications related to local tumor growth. Complete surgical resection was possible for only two of nine patients with embryonal carcinoma; both received adjuvant therapy and were alive and well 3 and 20 years later. Seven patients received radiation and/or chemotherapy but died of residual or metastatic disease. Successful treatment for children with embryonal carcinoma requires an operation aimed at either debulking or complete resection (if possible) coupled with early and aggressive combination chemotherapy. The role of radiation in primary therapy remains undefined with regard to curative intent.

Chemotherapy of mediastinal germ cell tumors.

P RIMARY GERM cell tumors of the mediastinum are rare. A recent review of 1687 patients with primary tumors and cysts of the mediastinum noted only 19 cases-an incidence slightly greater than 1% of all tumors in that anatomic region.’ In 1973, Rubush et al. reviewed 10 series and found 39 cases of malignant teratoma in 1037 patients with mediastinal tumors, an incidence of approximately 4%.* Though some mediastinal tumors may represent metastases from an occult or “burned out” testicular primary,3 careful postmortem evaluation of 20 patients with anterior mediastinal germ cell tumors supported the view that the great majority originate in the mediastinum.4 This is also reasonable from an embryologic point of view, since the urogenital ridge extends from the sixth cervical vertebra to the third lumbar vertebra. Because of the rarity of the disease, collected series from a single institution are uncommon. In 1974, Memorial Hospital reported 30 patients with primary mediastinal germ cell tumors,’ and 17 cases were reported in 1978 from the M. D. Anderson Hospital.h All histologic subtypes described in testicular cancer were found: seminoma, teratoma, embryonal carcinoma, and choriocarcinoma. The incidence in both series were similar: pure seminoma in 10 and 5 patients, pure embryonal carcinoma in 4 and 1, mixed tumors in 16 and 10, and pure choriocarcinema in 0 and I, respectively. Review of the literature reveals wide variation in histologic terminology, but the tumors can be broadly divided into seminomatous and nonseminomatous types. This separation also applies to their prognosis and treatment. Seminoma is the most common pure subtype as well as the most treatable. Surgery alone, or surgery plus radiation therapy, are effective in mediastinal seminoma, with reported long-term survival rates greater than SO%.‘.s.7~~9 Sterchi and Cordell, in their 1975 review, found an overall 5-yr survival rate of 58% in 105 patients, only 5 of whom were known to have received chemotherapy.’ While chemotherapy without irradiation is very uncommon as the single therapeutic modality, a patient with metastatic mediastinal