Teratoma With Somatic-Type Malignant Components in Germ Cell Tumors of the Testis: A Clinicopathologic Analysis of 40 Cases With Outcome Correlation

Abstract

Teratoma with a malignant somatic component (MSC) is a rare phenomenon recognized when a somatic type malignancy occurs in the context of a germ cell tumor (GCT). The authors present their 29-year experience with 40 patients treated from 1981 to 2009 in their institution. The average age was 31 years. All patients underwent radical orchiectomy, which demonstrated a GCT in 19 with an MSC. The MSC was observed in the other 21 cases in metastatic sites, including lung (13), liver (3), pleura (1), mediastinum (7), supraclavicular lymph nodes (1), and retroperitoneal lymph nodes (9). The most common histologic types were rhabdomyosarcoma (n = 11) and primitive neuroectodermal tumors (n = 10), followed by adenocarcinoma (n = 9), sarcoma, not otherwise specified (n = 5), well-differentiated liposarcoma (n = 2), leiomyosarcoma (n = 2), chondrosarcoma (n = 2), nephroblastoma (Wilms's tumor), carcinoid, malignant peripheral nerve sheath tumor, and a gemistiocytic astrocytoma with choroid plexus tumor (each n = 1). Clinical data were collected through a dedicated database and are available for all 40 cases. After a median follow-up of 37 months, 29/40 (72.5%) were alive and disease-free. Stage-specific survival was 100% for stage I, 87.5% for stage II, and 53% for stage III patients suggesting that patients with MSC confined to the testis have a good prognosis. A lower sensitivity to chemotherapy for a MSC when compared with typical GCT is reported. Surgical excision of the MSC is therefore an essential part of the management.

Germ Cell Tumors with Malignant Somatic Transformation: A Mayo Clinic Experience

“…Instead, grade of sarcoma, but not carcinoma, and diagnosis at late relapse were associated with cancer-specific mortality. Conversely, Colecchia et al [12 ]found stage at presentation to be a strong disease-related prognostic factor in their 40 cases. Lastly, a series of 33 cases by Guo et al [14 ]demonstrated that the presence of MST in metastasis is associated with higher rates of mortality.…”

“…Efforts to understand the prognostic factors and optimal management of this presentation of GCTs remain elusive as discussion is limited to case reports and a small number of published clinicopathologic series at referral centers [9,10,12,13,14,15,16]. To date, most studies have not shown significantly different survival outcomes between secondary somatic histologic subtypes [10,14,15].…”

Teratoma with somatic-type malignant components of the testis. A review and an update

“…It occurs in 3-6 % of patients with metastatic germ cell tumor (GCT). Further analyses have subsequently been reported, however, each one covering a small number of patients [5,7,10].…”

“…Clinical follow ups have shown that compared with teratomas without somatic type malignancies, these tumors in metastatic sites have poor prognosis if not completely resectable. The extent of the disease at onset and consequently, the feasibility of a radical surgical extirpation have been identified as the strongest prognostic parameters for durable remissions [3,[8][9][10].…”

“…Among this great morphological variety of different subtypes of germ cell malignancies, teratomas can show a partial transformation into a malignant somatic-type tumor component (MSC) [1][2][3]. However, due to the small number of published clinic pathological studies, our knowledge about these tumors is very limited [4][5][6][7][8][9][10].…”